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CASE REPORT
Year : 2019  |  Volume : 12  |  Issue : 4  |  Page : 370-372  

Oromandibular dystonia: A rare clinical entity


1 Department of Psychiatry, Dr. DY Patil Medical College, Dr. DY Patil Vidyapeeth, Pune, Maharashtra, India
2 Department of Neurology, Dr. DY Patil Medical College, Dr. DY Patil Vidyapeeth, Pune, Maharashtra, India

Date of Submission03-Aug-2018
Date of Acceptance16-Jan-2019
Date of Web Publication8-Jul-2019

Correspondence Address:
Suprakash Chaudhury
Department of Psychiatry, Dr. DY Patil Medical College, Dr. DY Patil Vidyapeeth, Pimpri, Pune - 411 018, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_123_18

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  Abstract 


Oromandibular dystonia (OMD) is a chronic, disabling focal dystonia involving masticatory, facial, pharyngeal, lingual, and lip muscles. OMD interferes with normal orofacial functions, such as deglutition and speech leading to impaired quality of life. Cosmetic disfiguration and social embarrassment faced by these patients owing to the perturbing nature of movements leads to anxiety, depression and social isolation. There are very few reported cases of OMD in Indian literature as the disorders are often misdiagnosed. We present two cases of OMD who were initially misdiagnosed but later responded well to appropriate therapy. A comprehensive evaluation and a holistic approach are crucial for the prompt management of OMD.

Keywords: Blepharospasm-plus syndrome, botulinum toxin type A, geste antagoniste, oromandibular dystonia


How to cite this article:
Shailaja B, Chaudhury S, Rohatgi S, Saldanha D. Oromandibular dystonia: A rare clinical entity. Med J DY Patil Vidyapeeth 2019;12:370-2

How to cite this URL:
Shailaja B, Chaudhury S, Rohatgi S, Saldanha D. Oromandibular dystonia: A rare clinical entity. Med J DY Patil Vidyapeeth [serial online] 2019 [cited 2019 Aug 18];12:370-2. Available from: http://www.mjdrdypv.org/text.asp?2019/12/4/370/262226




  Introduction Top


Oromandibular dystonia (OMD) is a disorder of movement affecting the lower part of the face and the jaw. It is characterized by the involuntary, paroxysmal, sustained, or intermittent movements of jaw and tongue with facial grimacing produced by spasms of the masticatory, facial, pharyngeal, lingual, and lip muscles of variable severity. These movements can be patterned, twisting, or tremulous. OMD is classified as jaw opening, jaw closing, jaw deviating, lingual, and mixed types. OMD presenting with blepharospasm once known as Brueghel syndrome or Meige's syndrome is currently included in blepharospasm-plus syndrome due to the imprecise nature of the eponyms. OMD generally appears at middle age or beyond. It is twice more common in women than in men. More than one-third of the patients with OMD have dysphagia, dysarthria, impaired salivation and few suffer from dental attrition, headache, and facial pains. Cosmetic disfiguration and social embarrassment faced by the patients owing to the perturbing nature of movements leads to anxiety, depression and social isolation thus hampering the quality of life. OMD is either primary reflecting a dysfunction in the basal ganglia-thalamo-cortical circuits or secondary to medications, essential tremors, Parkinson's disease, Huntington disease, neuroacanthosis, Wilson disease, Lesch-Nyhan syndrome, oromandibular-facial trauma, and ill-fitting dentures. Task-specific OMDs were seen in wind instrument players, auctioneers, and bingo callers. The most common secondary type is tardive dystonia, developing as a side effect of long-term treatment with antipsychotic drugs. Due to high-clinical variability and rare occurrence, OMD poses diagnostic and therapeutic challenges.[1],[2],[3],[4],[5],[6],[7],[8] In this case series, we present two cases of OMD.


  Case Reports Top


Case A

A 54-year-old female presented with gaping of mouth, spontaneous, repetitive, spasmodic movement of the lower jaw, facial grimaces, bouts of belching, frequent blinking and difficulty in opening eyes fully for the last 2 years. Due to the inability to close the mouth completely, she used to wrap a scarf tightly around her face to close the mouth. She used to have spontaneous, repetitive, painless movements of the lower jaw muscles throughout the day, and bouts of belching slowly subsiding over 5–6 h which were socially embarrassing. Though she could not exercise control over the jaw movements or belching, it used to subside to a certain extent, whenever she engaged herself in conversations. She used to find difficulty in swallowing and chewing due to which, liquids were preferred over solids. She would use the straw to drink. Over the span of 2 years, she had lost almost 8 kg of her body weight due to altered food habits. Her speech had several pauses and breaks. Although she could fetch a fairly good amount of sleep as movements used to temporarily disappear on falling asleep, sleep onset would often be delayed due to the troublesome nature of the jaw movements. She would blink often. At times, it would be difficult for her to open her eyes fully, so she would manually open her eyes. The aforementioned symptoms had an insidious onset and progressive course. Over the last 2 years, she had consulted multiple physicians and psychiatrists and was diagnosed with the dissociative motor disorder and was tried on a various combination of selective serotonin reuptake inhibitors, benzodiazepines and low-dose antipsychotic medication with no improvement.

Case B

A 76-year-old female presented with complaints of spontaneous, repetitive, painless spasmodic movements of lower jaw throughout the day, pursing of lips and bouts of belching lasting for about half an hour for the last 1½ year. Her speech had long pauses. The jaw movements used to subside to a certain extent whenever she would engage in conversations and would disappear completely with sleep. Despite multiple consultations with physicians over a year, there was no improvement leading to psychiatric referral.

In both the subjects, there was no significant stressor in temporal relation to the onset of illness. Bouts of belching experienced by the subjects were thought to be secondary to aerophagia caused by open mouth breathing. They were distressed by the burdensome nature of the symptoms, altered food habits, difficult articulation, cosmetic disfiguration, and social embarrassment secondary to it and were disappointed with the undue delay in the diagnosis and treatment. There were no primary or secondary gains. There was no appreciable drug history before the onset. No relevant past or family history of any medical/psychiatric illness could be noted. Premorbid personality was well adjusted. Mental Status Examination revealed distressed affect, and somatic preoccupation. Both the subjects were well oriented, alert, and attentive. Intelligence, memory, and abstraction were satisfactory with fair insight and judgment. Psychiatric evaluation did not reveal any psychiatric comorbidity. The cases were then referred to the neurologist for an opinion. The general physical examination was unremarkable. The neurological evaluation showed intact cranial nerves, coordination, motor and sensory functions. Ophthalmological examination, including slit-lamp biomicroscopy, was normal except for blepharospasm in case A. Routine investigations and serum calcium level were within the normal limit. Computed Tomography Scan and Magnetic Resonance Imaging revealed no neuroparenchymal abnormality. Case A was diagnosed with oromandibular dystonia and was given botulinum toxin type A injection and clonazepam 0.5 mg on SOS basis with which patient showed dramatic improvement within 2 days. This was assessed by the neurologist based on complaints and clinical examination. On review after a month, she remained symptom-free. Case B was diagnosed with oromandibular dystonia and was started on tetrabenazine 25 mg/day which was up-titrated to 50 mg/day in a week and trihexyphenidyl 4 mg/day with which 30% betterment was reported. She was lost to follow-up thereafter. Thus, in both the cases, a regular and long-term follow-up was not possible. Hence, the sustained benefit with treatment could not be ensured in these cases.


  Discussion Top


Few of the clinical features of the above-mentioned cases were suggestive of dissociative motor disorder such as movements subsiding, whenever patient engaged in conversation, temporary disappearance of symptoms with sleep. However, the symptoms of OMD can be worsened by emotional factors and can be relieved by sleeping, relaxing, and talking. The gesteantagoniste (GA) is a voluntary procedure that briefly mitigates the severity of dystonic movements. GA may either be a sensory trick, in which patients may temporarily reduce dystonia by gently touching the face or neck, biting the toothpick/lip or as a forcible trick, like applying counter-pressure in opposition to the dystonic head turn, may be necessary for severe forms of dystonia. The tactile/proprioceptive sensory gating modulating the sensorimotor integration and diminishing abnormal dystonic motor output explains the antidystonic effect of GA. Points against the diagnosis of dissociative disorder were the late age of onset, the absence of significant stressor in temporal relation with the onset, absence of gain and good premorbid personality. In addition, there was no account of the comorbid or history of psychiatric disorder. The diagnosis of OMD is based on history and clinical examination and there is no gold standard test to confirm the diagnosis. As a result, it is often misdiagnosed as a temporomandibular joint syndrome, hemifacial spasm or psychological disorder, and subsequently, there is a delay in treatment or patients are managed incorrectly. It is essential to exclude all secondary causes such as drugs, trauma, associated neurological disorders like Wilson's disease, etc. A neurological examination followed by an MRI must be performed to evaluate for stroke or space occupying lesion of the basal ganglia. Ceruloplasmin level and slit-lamp biomicroscopy to be carried out to rule out the possibility of Wilson's disease. Electrocardiogram and Oromandibular dystonia screening questionnaire may be of some assistance in the differentiation of oromandibular dystonia from other conditions.[9],[10],[11]

Oral medication is the first line of treatment. Oral antidystonic medication includes anticholinergic drugs, benzodiazepines, and anticonvulsants. However, the effects are variable. Then, next line of treatment is injections of botulinum A, though the effects are temporary, rapid symptom relief and high response rate of 90%–95% is reported. The GA can be used as an adjuvant to pharmacotherapy. Local anesthetic blocks, speech therapy, and the use of oral sensory devices constitute other therapeutic options for OMD. More recently, deep brain stimulation of bilateral globus pallidus interna has emerged as a therapeutic option in patients with intractable OMD.[3],[12],[13],[14],[15],[16] As patients with OMD face social, emotional, and nutritional problems, a holistic approach is crucial. Antidepressant medication and supportive psychotherapy benefit patients suffering from depression and anxiety. Nutritional issues can be resolved by the use of texture modified diets, nutritional supplement drinks, and food fortification.[17]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Maestre-Ferrín L, Burguera JA, Peñarrocha-Diago M, Peñarrocha-Diago M. Oromandibular dystonia: A dental approach. Med Oral Patol Oral Cir Bucal 2010;15:e25-7.  Back to cited text no. 12
    
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Tintner R, Jankovic J. Botulinum Toxin type A in the Management of Oromandibular Dystonia and Bruxism. Scientific and Therapeutic Aspects of Botulinum Toxin. Philadelphia: Lippincott Williams & Wilkins; 2002. p. 343-50.  Back to cited text no. 13
    
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Pandey S, Sharma S. Meige's syndrome: History, epidemiology, clinical features, pathogenesis and treatment. J Neurol Sci 2017;372:162-70.  Back to cited text no. 16
    
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